RETROPERITONEAL ROUND-CELL LIPOSARCOMA ASSOCIATED WITH PARANEOPLASTICPEMPHIGUS PRESENTING AS LICHEN-PLANUS PEMPHIGOIDES-LIKE ERUPTION

A 59-year old Caucasian woman refugee from Bosnia had been in good hea lth until a year before admission, when she developed painful erosions and ulcerated lesions in the mouth, anogenital area, hands, and feet. Treatment with topical and systemic antibiotics, acyclovir, and antif ungal creams was ineffective. Three months before admission, her skin and mucosal lesions had worsened, resulting in the limitation of walki ng and her daily activities: She had also experienced significant weig ht loss. On admission to the Department of Dermatology, University of Belgrade, the patient was noted to be thin, distressed, and quite depr essed. Physical examination revealed ulceration that involved the floo r of the mouth, ventral and lateral tongue, buccal mucosa, and hard an d soft palate (Fig. 1). The vermilion surfaces were fissured, eroded, and crusted with shiny violaceous scaly papules dispersed across the c utaneous edge of both upper and lower lip (Fig. 1). Skin examination d isclosed scattered violaceous scaly lesions on the flank, wrists, dors a of the hands and feet, and lower legs. There were numerous erosions with livid erythema on the palms and soles, fingers and toes, with fis sured nails, partial and total onycholysis, and pterygium formation. O n the vulvar and vaginal mucosa, as well as the anal acid perianal are a, numerous erosions with erythema and white exudate were also present . On the skin of the anterior abdomen, both axillas, and right groin, large tense blisters were noticed. In the right lower quadrant of the abdomen, a firm, painful, ill-defined mass was palpated, approximately 15 cm below the right costal margin. A pelvic examination confirmed a hard, immobile, painful, abdominal mass, with the suggested origin fr om the right adnexal (tubo-ovarial) region. Abdominal and pelvic sonog ram, as well as CT scan revealed a 10 x 9 cm, right-sided retroperiton eal tumor. Chest rentgenogram, and CT scan of the lungs were within no rmal limits. Laboratory studies revealed normocytic, normochromic anem ia with hypoalbuminemia. The following analyses were negative or withi n normal limits: antinuclear antibodies, cryoglobulins, VDRL, cultures for candida and Herpes virus from oral and perianal mucosa. A biopsy specimen from the oral mucosa demonstrated acantholytic blister with k eratinocyte necrosis and vacuolar interface changes. A biopsy of the v iolaceous papules from the wrist, finger and lower legs showed hyperke ratosis, hypergranulosis, dyskeratotic keratinocytes, prominent vacuol ar interlace changes, lichenoid infiltrate with exocytosis of mononucl ear cells and superficial, subcorneal clefting in one specimen (Fig. 2 ). Biopsy of the tense blister from the anterior abdomen disclosed sub epidermal bulla consistent with bullous pemphigoid. Direct immunofluor escence (DIF) of perilesional skin and oral mucosa revealed intercellu lar binding of IgG as well as diffuse deposition of IgG and C-3 along with dermoepidermal junction. Indirect immunofluorescence (IIF) studie s of the patient serum revealed strong intercellular binding on human skin (titer 1:640) as well as on transitional epithelium of rodent uri nary bladder (titer 1:40) (Pig. 3). Immunoprecipitation studies were n ot available at the time of hospitalization and were not performed.